Introduction: Primary squamous cell carcinoma (SCC) of the ampulla of Vater is an exceptionally rare malignancy, with only a limited number of cases reported in the literature. Its pathogenesis, biological behavior, and optimal management remain poorly defined. Case presentation: We report the case of a 68-year-old man presenting with several weeks of atypical abdominal discomfort and cholestatic liver enzyme abnormalities. Upper gastrointestinal endoscopy revealed an ulcerated ampullary lesion, and biopsy confirmed moderately to poorly differentiated SCC. Cross-sectional imaging excluded metastatic disease. The patient underwent pylorus-preserving pancreaticoduodenectomy. Histopathological analysis confirmed primary SCC (pT2N0M0) with negative margins (R0 resection). Adjuvant chemotherapy with cisplatin and 5-fluorouracil was initiated 6 weeks postoperatively, with a planned duration of 6 months. The patient remains disease-free at 6-month follow-up. Discussion: Ampullary SCC is rare but not unique, with several cases reported. Surgical resection remains the cornerstone of treatment. Adjuvant therapy is extrapolated from other SCCs due to a lack of evidence. Prognosis appears variable, with better outcomes in node-negative disease. Conclusion: This case highlights the importance of accurate histopathological diagnosis and reinforces the role of radical surgical resection. Further case accumulation is required to define optimal management strategies.
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